Amyloisosis

Question 1

Defin

Answer 1

EC deposits of a prot in abn fibrillar form, resis ot degrad.
-primary amyloisosis-
Prolif of plasma cell clone, amyloidogenic MC Igs, fibrillar light chain prot deposit, organ fail.
Kidn glom lesions
Cardiomyop, arrhyth, angina
Periph and ANS neuropathy, CTS
Macroglossia, malabs, wl, perforat, haemorr, obstruc, hepatosplenomeg.
Purpura.
Mx-nutrit, prednisolone and melphalan, SC transpl.
-secondary amyloidosis-
Amyloid derived from serum amyloid A an acute phase prot due to chronic inflamm eg RA, crohns, chronic infec.
Affs liver, kidn, spleen.
Px- proteinuria, nephrotic synd, hepatosplenomeg.
-familial-
Sensory or ANS neuropathy. Renal and heart inv. liver transpl can cure.

Question 2

Investig

Answer 2

Biop

Pos congo red and red green ploarised LM